biliary talk final
TRANSCRIPT
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Biliary Tract Disease
August 26, 2014
V. Raman Muthusamy, MD, FACG, FASGEDirector of Interventional Endoscopy
Clinical Professor of MedicineDavid Geffen School of Medicine at UCLA
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Gallstones
• Common: about 20-30 million in US• Gender and Ethnic Predisposition- Over 65% are female- Hispanic and Native American
• Lower socioeconomic status• 700,000 annual cholecystectomies in
U.S.• $6.5 billion annual cost
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Ultrasound Images
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Natural History of Gallstones
• Mostly asymptomatic: 70-90%• 2-4% develop symptoms annually- 10-20% with symptoms have CBD stones
• If symptomatic, frequently recurs (40%)• Gracie and Ransohoff- 5 yrs: 10% symptomatic
- 10 yrs: 15% symptomatic- 15 yrs: 18% symptomatic- No sig complications
Ann Intern Med 1983;99:199-205
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Types of Gallstones
• Cholesterol
- Most Common (75%)
• Pigmented: usually black
- 20% stones
• Brown
- More common in Asia
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Stones
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Cholesterol stones
• Obese women• Rapid weight loss: RYGB, lap band• Terminal ileal resection or disease- Crohn’s disease
• Pregnancy: saturation and poor motility• Drugs: estrogen, OCPs, TPN, octreotide• Genetics• Hypertriglyceridemia, DM, ? vagotomy
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Cholesterol Gallstone
• Cholesterol saturation and Dysmotility• Nucleation- Calcium nidus- Defective acidification- Nucleation vs. Anti-nucleation factors
• Growth- Gallbladder dysmotility (TPN,
pregnancy, octreotide)- Gallbladder mucus
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Pigmented Gallstones
• Bilirubin deconjugation and precipitation
- Bacterial β glucuronidase
• Chronic hemolytic disorders
- Sickle cell, thalassemias
• Chronic liver disease: ie. Cirrhosis
• Gallbladder stasis• Older age and women
• Disrupted enterohepatic circulation
- TI resection, Crohn’s disease
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Brown stones
• Seen with chronic infections of biliary tree
- Bacterial and parasitic• Asian-pacific ethnicity
• Prior surgery of biliary tree
- Stasis and dilation of duct system
- Often after cholecystectomy
• Recurrent pyogenic cholangiopathy
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Gallbladder Sludge
• Mixture of crystals and mucus
• Can cause the same symptoms as stones• Can be transient
• Diagnosed on ultrasound
• Nonshadowing
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KUB of calcified gallstones
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CT scan of gallstones
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Complications of Cholelithiasis
• Biliary colic• Cholecystitis
• Fistulization (GB to duodenum)• Mirizzi syndrome
• Cholangitis
• Pancreatitis
• GB cancer
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Biliary Colic
• RUQ, mid-epigastric• Lasts hours not weeks
- Crescendo, plateau, and then relief• Often post-prandial
- Nocturnal awakening
- Many hours after meal
• Nausea and vomiting
• Radiation to shoulder and back
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Acute Cholecystitis
• Duration: hours to days• Cystic duct obstruction
• HIDA scan: Sens/Spec > 90%• Ultrasonography: Sens/Spec > 90%
• Alk phos, transaminases rarely twice normal
• Mortality: 5-10% advanced age, co-morbidities, complications
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Acute Cholecystitis Complications
• Emphysematous cholecystitis
- Gangrenous gallbladder
• Perforation• Mirizzi Syndrome
- Cystic duct stone leading to biliary obstruction
• Cholecystoenteric fistula
- Pneumobilia
- Duodenum, Colon, Jejunum, Stomach
- Gallstone ileus
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Mirizzi’s Syndrome
Cystic Duct Stone Compressing CBD
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Cholescintigraphy (HIDA)False Positives
• Prolonged fasting
• Liver disease
- Tracer absorbed too slowly
• Chronic cholecystitis
• Total parenteral nutrition• Critical illness
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Board Questions
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76 yo male
• In ICU for recent STEMI, moderate failure• On ventilator, stormy course; NPO,
distended abdomen
• No prior abdominal surgeries• Low grade fever, leukocytosis
• Tenderness in RUQ, no rebound• Increasing AST, ALT and alk phos
- Bilirubin normal
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What is the most likely diagnosis??
A. Mirizzi’s syndrome
B. Choledocholithiasis
C. Ascending cholangitis
D. Acalculus cholecystitis
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What is the best therapy in the critically ill patient??
A. Lap cholecystectomy
B. ERCP with sphincterotomy
C. Percutaneous cholecystotomy
D. Observation
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Acalculous Cholecystitis
• Older; male > female• Triad
- Hemodynamic instability
- Prolonged fasting
- Immobility
• Fever, RUQ pain, leukocytosis, tenderness may be absent
• More fulminant course
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Acalculous Cholecystitis
• RUQ sonogram- GB wall > 4 mm*
- Sonographic Murphy’s sign
- Pericholecystic fluid collection
- Sensitivity: 67-92%
- Specificity: > 90%
• CT scan- GB wall > 4 mm
- Subserosal edema
- Intramural gas
- Sloughed mucosa
- Sens / Spec >95%
• Scintigraphy- False (+) and (-)
NB. Ascites without other cause
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Acalculous Cholecystitis
• Treatment- Urgent
cholecystectomy
- Percutaneous drainage
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Cholecystectomy
• Open or laparoscopic: 0.1% mortality
• Laparoscopic: reduction in postoperative LOS and pain
• Complicated presentation, coagulopathy or adhesions->open
• IOC: allows for ID and potential treatment of choledocholithiasis
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Complications of Cholecystectomy
• Overall incidence: ~1% in recent series• Types:
- Bile leak without significant duct injury
- Major duct injury +/- leak• Transection, stricture etc
• Typically treat leaks with ERCP with stent placement for 4-6 weeks without sphincterotomy (90% success rate)
• Endoscopic therapy similar to surgery for major biliary injury except for disconnection
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ERCP with Stent placement for CBD Leak
Before After (Healed)
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Other Treatment Modalities
• Ursodeoxycholic acid
- Patent cystic duct and non-calcified stones
- Small floating stones best response
- High recurrence rate (>50%)
- Risk of pancreatitis and cholangitis• ESWL not approved in U.S.
• Cholecystostomy tube
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Gallbladder Dyskinesia
• Decreased GB ejection (via CCK-HIDA) fraction may predispose to gallstone formation
• Controversial whether cholecystectomy leads to symptomatic improvement
• More common in subjects with functional bowel disorders
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Gallbladder Polyps
• Common: 1-5% of population• > 95% non-neoplastic (mostly cholesterol)
• Follow with ultrasound for 6 month-1 yr intervals
• If growth > 10 mm, cholecystectomy
• If no growth after 2 yrs, can dispense surveillance
• GB polyp (any size) + PSC = surgery
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Gallbladder Polyps
• Cholesterolosis: Most common (60%)
• Adenomyomatosis (25%)
• Inflammatory (10%)
• Adenoma: rare
- May be precursor to GB cancer
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Gallbladder Polyps
• Cholesterolosis
- Cholesterol / TG within epithelial macrophages
- Local or diffuse
- Can be combined with cholesterol polyps
- 10-15% autopsy series
- Ultrasound remains best way to detect
- Rarely thought to be symptomatic
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Cholesterol vs adenomatous polyp
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Gallbladder Polyps
• Adenomyomatosis
- Hypertrophy of the muscle layer
- Usually focal (fundus) although can be diffuse
- Incidental and asymptomatic
- Classic comet tail appearance on US
- Surgery when wall > 10 mm, associated mass or in rare instances with diffuse disease
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“Comet sign” of Adenomyomytosis
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GB Cancer
Incidence: 5000/yr in US; 1-2% of GB surg
Late diagnosis commonSurgery only curative option
- Radical Cholecystectomy
5 yr survival: <10%Associations:
- Female gender, Caucasian race, increasing age- Porcelain gallbladder- PSC and IBD- Anomalous pancreaticobiliary junction (APBJ)- Cholelithiasis- Chronic Salmonella typhi- Adenomatous GB polyps
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Gallbladder Cancer
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Choledocholithiasis
• Can present with biliary colic
• Elevated liver profile • Dilated common bile duct: when chronic
• Jaundice
• Ascending Cholangitis• Pancreatitis
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Ascending Cholangitis• Infection of CBD: Emergency
- Duct usually obstructed: stones, stent, parasite
- Abnormal duct: post-op, stricture, anastomosis
• Usually bacterial: E coli: 25-50%
- Klebsiella: 15-20%; Enterobacter: 5-10%
- Enterococcus: 10-20%• Treatment: Resuscitate
- Antibiotics: Broad spectrum (if ill, cover enterococcus)
- Relieve obstruction: ERCP, perc drain, surgery
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Ascending Cholangitis: 15-50%
Charcot’sTriad
Fever
RUQ Pain
Jaundice
Reynold’s Pentad: Add MS changes, shock
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Choledocholithiasis
• Recurrent biliary colic in the post-cholecystectomy patient
• ERCP for patients with high probability
- 95% success rate
- 5% complication rate
• For low or intermediate probability
- MRCP – usually for low probability
- EUS – often for intermediate probability
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Choledocholithiasis
• Biliary sphincterotomy
• Balloon / basket extraction
• Balloon sphincteroplasty
- For larger stones; usu after small sphincterotomy
• Lithotripsy
- Mechanical (basket)
- Cholangioscopy
• Laser
• Electrohydraulic (EHL)
- Extracorporeal shockwave lithotripsy
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Sphincter of Oddi Dysfunction
• Stenosis or spasm of the Sphincter of Oddi• Persistent / recurrent biliary pain following
cholecystectomy without structural abnormalities
• Idiopathic recurrent pancreatitis- Recent study shows no advantage to dual
sphincterotomy over biliary sphx alone
• Biliary type pain with intact gallbladder and no stones
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Sphincter of Oddi Dysfunction
• Type I:
- biliary type pain, elevated liver enzymes (> 2x nl on two occasions)
- bile duct diameter > 12 mm on ERCP
- delayed contrast drainage > 45 minute• Type II: biliary type pain and 1 or 2 of the
aforementioned criteria
• Type III: biliary type pain only
Rome III criteria: Doesn’t include contrast drainage as a criteria.
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Sphincter of Oddi Dysfunction
• Type I: empiric biliary sphincterotomy
• Type II: sphincter of Oddi manometry (SOM)
- Abnormal in up to 50-63% pts
- Pain relief up to 85% in this subset with biliary sphincterotomy
- Normal SOM and sphincterotomy: controversial
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Sphincter of Oddi Dysfunction
• Type III:
- Less than 10% response to sphincterotomy
- NIH sponsored trial recently published in JAMA shows NO benefit for manometric testing and sphincterotomy
- ERCP +/- SOM no longer recommended
- Consider other treatments for functional bowel disorders
Cotton et al, JAMA 2014, 311:(20)
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Sphincter of Oddi Manometry
Basal pressure > 40 mm HgCorrelates best with presence
of symptoms and relief following sphincterotomy
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Choledochal Cysts
• Congenital anomalies of the biliary tree
• Incidence: 1/100-150,000• 15-100 x more common in Japan
• Females more commonly affected (3-4:1)
• 2/3 cases manifest by age 10• More seen in adults recently
• Classic triad: Abd. Pain, jaundice, palpable mass
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Choledochal Cysts• Type I: (most common) 80-90% of cases.
Fusiform dilation of CBD (segmental or diffuse)
• Type II: Choledochal diverticulum: 2%• Type III: Choledochocele (intraduodenal
dilation): 1-5%• Type IV: Multiple extra +/- intrahepatic
cysts
- 10%, divided into and A and B type• Type V: Multiple intrahepatic dilation
(Caroli’s disease): rare, but up to 20% in some series
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Choledochal Cysts• Association with anomalous union of pancreatic
and biliary ducts (seen in 50-80%)- Recommend cholecystectomy for GB CA risk
• Jaundice, abdominal pain, intraductal stones• At risk for cholangiocarcinoma (esp. I and IV)
- Risk of 10-30%; 20-30 x gen. population
• Surgical excision for most cysts- Type III: Large biliary sphincterotomy
• Caroli's disease associated with congenital hepatic fibrosis, renal tubular ectasia and polycystic kidney disease
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Todani classification
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Board Questions
• 39 yo male presents with pruritis, jaundice and pain
• No prior PMH or PSH• FH is non-contributory• PE: scleral icterus, mid-
epigastric tenderness• LABS: Alk phos: 487,• Bili: 5.2, AST: 164, ALT:
628• CT on right panel
revealed no stones:
Coyle in GASTRO 2010;138:e3–e4
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Board Question
• Endoscopy reveals bulge at ampulla
Coyle in GASTRO 2010;138:e3–e4
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Board Question 1: What is the most likely diagnosis?
• A. Choledocholithiasis• B. Peri-ampullary diverticulum
• C. Choledochal Cyst• D. Ampullary adenoma
• E. None of the above
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Board Question 2: What is the best treatment?
• A. Observation• B. Ampullectomy
• C. Whipple resection• D. Sphincterotomy
• E. None of the above
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Board Question Answer
• This is a choledochal cyst, Type III
• Aka Choledochocele• Can usually be
treated by large, biliary sphincterotomy that unroofs the cyst
Coyle in GASTRO 2010;138:e3–e4
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Summary
• Stone disease: Common- Know the types and pathogenesis- Know the complications and treatment
• Gallbladder polyps- Know the types and natural history
• Acalculous cholecystitis- Recognize and treat
• Choledocholithiasis:- Know how to diagnose and treat
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Summary
• Ascending Cholangitis: Recognize, urgent treatment- Know organisms and causes
• Sphincter of Oddi dysfunction
- Know sub-types and treatment• Choledochal cysts- Know types, natural history and
treatment